TY - EJOU
AU - An, Yong
AU - Wang, Gang
AU - Dai, Jiangtao
AU - Pan, Zhengxia
AU - Wu, Yuhao
TI - Reverse Potts Shunt in Children with Suprasystemic Pulmonary Arterial Hypertension: A Systematic Review and Meta-Analysis
T2 - Structural and Congenital Heart Disease
PY - 2025
VL - 20
IS - 1
SN - 3071-1738
AB - Background Pulmonary arterial hypertension (PAH) is a progressive condition with a poor prognosis in children. Lung transplantation (Ltx) remains the ultimate option when patients are refractory to PAH-specific therapy. Reverse Potts shunt (RPS) has been introduced to treat suprasystemic PAH. This study aims to investigate the clinical outcomes of suprasystemic PAH in children. Methods Embase, Pubmed, and the Cochrane Library databases were searched for related studies that reported the clinical outcomes of suprasystemic PAH following RPS in children. To investigate the clinical outcomes of RPS, meta-analyses of the early and overall mortalities were performed. Results Nine studies were included in this study. The estimated early mortality was 14.4% (95% CI, 7.1% to 23.1%), and the overall mortality/Ltx was 23.2% (95% CI, 14.4% to 32.9%). The estimated 1-year survival was 86.3% (95% CI, 75.9% to 88.7%). A qualitative review showed that the median value of 5-year survival free from Ltx of patients undergoing RPS was 68.6% (range: 65% to 92.3%). Compared to Ltx, RPS did not significantly increase the early mortality (OR, 2.48, 95% CI 0.75 to 8.24, p = 0.14). RPS also significantly improved the New York Heart Association/World Health Organization functional class, reduced the BNP/NT-pro BNP levels, decreased the PAH-specific therapy, and increased the six-minute-walking distance. Conclusions RPS may serve as an alternative treatment for suprasystemic drug-refractory PAH. Further large-scale and prospective cohort studies are needed to validate these findings.
KW - Children; pulmonary arterial hypertension; potts shunt; outcomes; survival
DO - 10.32604/chd.2025.063152